Anomalous Origin of the Coronary Artery from the Pulmonary Artery in Children and Adults: A Pictorial Review of Cardiac Imaging Findings

Anomalous origin of the coronary artery from the pulmonary artery is a rare and potentially fatal congenital heart defect. Up to 90% of infants with an anomaly involving the left coronary artery die within the first year of life if left untreated. Patients who survive beyond infancy are at risk of sudden cardiac death. Cardiac CT and MRI are increasingly being used for the accurate diagnosis of this anomaly for prompt surgical restoration of the dual coronary artery system. Moreover, life-long imaging surveillance after surgery is necessary for these patients. In this pictorial review, multimodal cardiac imaging findings of this rare and potentially fatal coronary artery anomaly are comprehensively discussed, and representative images are provided to facilitate the understanding of this anomaly.

Right Ventricular Mass Quantification Using Cardiac CT and a Semiautomatic Three-Dimensional Hybrid Segmentation Approach: A Pilot Study

Objective@#To evaluate the technical applicability of a semiautomatic three-dimensional (3D) hybrid CT segmentation method for the quantification of right ventricular mass in patients with cardiovascular disease. @*Materials and Methods@#Cardiac CT (270 cardiac phases) was used to quantify right ventricular mass using a semiautomatic 3D hybrid segmentation method in 195 patients with cardiovascular disease. Data from 270 cardiac phases were divided into subgroups based on the extent of the segmentation error (no error; ≤ 10% error; > 10% error [technical failure]), defined as discontinuous areas in the right ventricular myocardium. The reproducibility of the right ventricular mass quantification was assessed. In patients with no error or 0.1). @*Conclusion@#CT quantification of right ventricular mass using a semiautomatic 3D hybrid segmentation is technically applicable with high reproducibility in most patients with cardiovascular disease.

Pediatric Cardiothoracic CT Guideline Provided by the Asian Society of Cardiovascular Imaging Congenital Heart Disease Study Group: Part 2. Contemporary Clinical Applications

The use of pediatric cardiothoracic CT for congenital heart disease (CHD) was traditionally limited to the morphologic evaluation of the extracardiac thoracic vessels, lungs, and airways. Currently, the applications of CT have increased, owing to technological advancements in hardware and software as well as several dose-reduction measures. In the previously published part 1 of the guideline by the Asian Society of Cardiovascular Imaging Congenital Heart Disease Study Group, we reviewed the prerequisite technical knowledge for clinical applications in a user-friendly and vendor-specific manner. Herein, we present the second part of our guideline on contemporary clinical applications of pediatric cardiothoracic CT for CHD based on the consensus of experts from the Asian Society of Cardiovascular Imaging CHD Study Group. This guideline describes up-to-date clinical applications effectively in a systematic fashion.

Anomalous Origin of the Coronary Artery from the Pulmonary Artery in Children and Adults: A Pictorial Review of Cardiac Imaging Findings

Anomalous origin of the coronary artery from the pulmonary artery is a rare and potentially fatal congenital heart defect. Up to 90% of infants with an anomaly involving the left coronary artery die within the first year of life if left untreated. Patients who survive beyond infancy are at risk of sudden cardiac death. Cardiac CT and MRI are increasingly being used for the accurate diagnosis of this anomaly for prompt surgical restoration of the dual coronary artery system. Moreover, life-long imaging surveillance after surgery is necessary for these patients. In this pictorial review, multimodal cardiac imaging findings of this rare and potentially fatal coronary artery anomaly are comprehensively discussed, and representative images are provided to facilitate the understanding of this anomaly.

Right Ventricular Mass Quantification Using Cardiac CT and a Semiautomatic Three-Dimensional Hybrid Segmentation Approach: A Pilot Study

Objective@#To evaluate the technical applicability of a semiautomatic three-dimensional (3D) hybrid CT segmentation method for the quantification of right ventricular mass in patients with cardiovascular disease. @*Materials and Methods@#Cardiac CT (270 cardiac phases) was used to quantify right ventricular mass using a semiautomatic 3D hybrid segmentation method in 195 patients with cardiovascular disease. Data from 270 cardiac phases were divided into subgroups based on the extent of the segmentation error (no error; ≤ 10% error; > 10% error [technical failure]), defined as discontinuous areas in the right ventricular myocardium. The reproducibility of the right ventricular mass quantification was assessed. In patients with no error or 0.1). @*Conclusion@#CT quantification of right ventricular mass using a semiautomatic 3D hybrid segmentation is technically applicable with high reproducibility in most patients with cardiovascular disease.

Pediatric Cardiothoracic CT Guideline Provided by the Asian Society of Cardiovascular Imaging Congenital Heart Disease Study Group: Part 2. Contemporary Clinical Applications

The use of pediatric cardiothoracic CT for congenital heart disease (CHD) was traditionally limited to the morphologic evaluation of the extracardiac thoracic vessels, lungs, and airways. Currently, the applications of CT have increased, owing to technological advancements in hardware and software as well as several dose-reduction measures. In the previously published part 1 of the guideline by the Asian Society of Cardiovascular Imaging Congenital Heart Disease Study Group, we reviewed the prerequisite technical knowledge for clinical applications in a user-friendly and vendor-specific manner. Herein, we present the second part of our guideline on contemporary clinical applications of pediatric cardiothoracic CT for CHD based on the consensus of experts from the Asian Society of Cardiovascular Imaging CHD Study Group. This guideline describes up-to-date clinical applications effectively in a systematic fashion.

Hydrocephalus: Ventricular Volume Quantification Using Three-Dimensional Brain CT Data and Semiautomatic Three-Dimensional Threshold-Based Segmentation Approach

Objective@#To evaluate the usefulness of the ventricular volume percentage quantified using three-dimensional (3D) brain computed tomography (CT) data for interpreting serial changes in hydrocephalus. @*Materials and Methods@#Intracranial and ventricular volumes were quantified using the semiautomatic 3D thresholdbased segmentation approach for 113 brain CT examinations (age at brain CT examination ≤ 18 years) in 38 patients with hydrocephalus. Changes in ventricular volume percentage were calculated using 75 serial brain CT pairs (time interval 173.6 ± 234.9 days) and compared with the conventional assessment of changes in hydrocephalus (increased, unchanged, or decreased).A cut-off value for the diagnosis of no change in hydrocephalus was calculated using receiver operating characteristic curve analysis. The reproducibility of the volumetric measurements was assessed using the intraclass correlation coefficient on a subset of 20 brain CT examinations. @*Results@#Mean intracranial volume, ventricular volume, and ventricular volume percentage were 1284.6 ± 297.1 cm 3 , 249.0 ± 150.8 cm 3 , and 19.9 ± 12.8%, respectively. The volumetric measurements were highly reproducible (intraclass correlation coefficient = 1.0). Serial changes (0.8 ± 0.6%) in ventricular volume percentage in the unchanged group (n = 28) were significantly smaller than those in the increased and decreased groups (6.8 ± 4.3% and 5.6 ± 4.2%, respectively; p = 0.001 and p < 0.001, respectively; n = 11 and n = 36, respectively). The ventricular volume percentage was an excellent parameter for evaluating the degree of hydrocephalus (area under the receiver operating characteristic curve = 0.975; 95% confidence interval, 0.948–1.000; p < 0.001). With a cut-off value of 2.4%, the diagnosis of unchanged hydrocephalus could be made with 83.0% sensitivity and 100.0% specificity. @*Conclusion@#The ventricular volume percentage quantified using 3D brain CT data is useful for interpreting serial changes in hydrocephalus.

CT and MRI for Repaired Complex Adult Congenital Heart Diseases

An increasing number of adult congenital heart disease (ACHD) patients continue to require life-long diagnostic imaging surveillance using cardiac CT and MRI. These patients typically exhibit a large spectrum of unique anatomical and functional changes resulting from either single- or multi-stage palliation and surgical correction. Radiologists involved in the diagnostic task of monitoring treatment effects and detecting potential complications should be familiar with common cardiac CT and MRI findings observed in patients with repaired complex ACHD. This review article highlights the contemporary role of CT and MRI in three commonly encountered repaired ACHD: repaired tetralogy of Fallot, transposition of the great arteries after arterial switch operation, and functional single ventricle after Fontan operation.

Double Outlet Right Ventricle: In-Depth Anatomic Review Using Three-Dimensional Cardiac CT Data

Double outlet right ventricle (DORV) is a relatively common congenital heart disease in which both great arteries are connected completely or predominantly to the morphologic RV. Unlike other congenital heart diseases, DORV demonstrates various anatomic and hemodynamic subtypes, mimicking ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, and functional single ventricle. Because different surgical strategies are applied to different subtypes of DORV with ventricular septal defects, a detailed assessment of intracardiac anatomy should be performed preoperatively. Due to high spatial and contrast resolutions, cardiac CT can provide an accurate characterization of various intracardiac morphologic features of DORV. In this pictorial essay, major anatomic factors affecting surgical decision-making in DORV with ventricular septal defects were comprehensively reviewed using three-dimensional cardiac CT data. In addition, the surgical procedures available for these patients and major postoperative complications are described.

Imaging Findings of Coronary Artery Fistula in Children: A Pictorial Review

Coronary artery fistula, defined as an abnormal communication between the coronary arteries and a cardiac chamber (most commonly) or a thoracic great vessel, may result in hemodynamically significant problems due to vascular shunting in children. Echocardiography, cardiac catheterization, cardiac MRI, and cardiac CT may be used to evaluate coronary artery fistula in children. Recently, CT has played a pivotal role for the accurate diagnosis of coronary artery fistula in children. Surgical or interventional treatment is performed for hemodynamically significant coronary artery fistulas. In this pictorial review, the detailed imaging findings of coronary artery fistula in children are described.

Pattern Analysis of Left Ventricular Remodeling Using Cardiac Computed Tomography in Children with Congenital Heart Disease: Preliminary Results

Objective@#To assess left ventricular remodeling patterns using cardiac computed tomography (CT) in children with congenital heart disease and correlate these patterns with their clinical course. @*Materials and Methods@#Left ventricular volume and myocardial mass were quantified in 17 children with congenital heart disease who underwent initial and follow-up end-systolic cardiac CT studies with a mean follow-up duration of 8.4 ± 9.7 months. Based on changes in the indexed left ventricular myocardial mass (LVMi) and left ventricular mass-volume ratio (LVMVR), left ventricular remodeling between the two serial cardiac CT examinations was categorized into one of four patterns: pattern 1, increased LVMi and increased LVMVR; pattern 2, decreased LVMi and decreased LVMVR; pattern 3, increased LVMi and decreased LVMVR; and pattern 4, decreased LVMi and increased LVMVR. Left ventricular remodeling patterns were correlated with unfavorable clinical courses. @*Results@#Baseline LVMi and LVMVR were 65.1 ± 37.9 g/m2 and 4.0 ± 3.2 g/mL, respectively. LVMi increased in 10 patients and decreased in seven patients. LVMVR increased in seven patients and decreased in 10 patients. Pattern 1 was observed in seven patients, pattern 2 in seven, and pattern 3 in three patients. Unfavorable events were observed in 29% (2/7) of patients with pattern 1 and 67% (2/3) of patients with pattern 3, but no such events occurred in pattern 2 during the follow-up period (4.4 ± 2.7 years). @*Conclusion@#Left ventricular remodeling patterns can be characterized using cardiac CT in children with congenital heart disease and may be used to predict their clinical course.

Optimal Attenuation Threshold for Quantifying CT Pulmonary Vascular Volume Ratio

Objective@#To evaluate the effects of attenuation threshold on CT pulmonary vascular volume ratios in children and young adults with congenital heart disease, and to suggest an optimal attenuation threshold. @*Materials and Methods@#CT percentages of right pulmonary vascular volume were compared and correlated with percentages calculated from nuclear medicine right lung perfusion in 52 patients with congenital heart disease. The selected patients had undergone electrocardiography-synchronized cardiothoracic CT and lung perfusion scintigraphy within a 1-year interval, but not interim surgical or transcatheter intervention. The percentages of CT right pulmonary vascular volumes were calculated with fixed (80–600 Hounsfield units [HU]) and adaptive thresholds (average pulmonary artery enhancement [PAavg] divided by 2.50, 2.00, 1.75, 1.63, 1.50, and 1.25). The optimal threshold exhibited the smallest mean difference, the lowest p-value in statistically significant paired comparisons, and the highest Pearson correlation coefficient. @*Results@#The PAavg value was 529.5 ± 164.8 HU (range, 250.1–956.6 HU). Results showed that fixed thresholds in the range of 320–400 HU, and adaptive thresholds of PAavg/1.75–1.50 were optimal for quantifying CT pulmonary vascular volume ratios. The optimal thresholds demonstrated a small mean difference of ≤ 5%, no significant difference (> 0.2 for fixed thresholds, and > 0.5 for adaptive thresholds), and a high correlation coefficient (0.93 for fixed thresholds, and 0.91 for adaptive thresholds). @*Conclusion@#The optimal fixed and adaptive thresholds for quantifying CT pulmonary vascular volume ratios appeared equally useful. However, when considering a wide range of PAavg, application of optimal adaptive thresholds may be more suitable than fixed thresholds in actual clinical practice.

Advanced Medical Use of Three-Dimensional Imaging in Congenital Heart Disease: Augmented Reality, Mixed Reality, Virtual Reality, and Three-Dimensional Printing

Quantification of Initial Right Ventricular Dimensions by Computed Tomography in Infants with Congenital Heart Disease and a Hypoplastic Right Ventricle

Four-Dimensional Thoracic CT in Free-Breathing Children

In pediatric thoracic CT, respiratory motion is generally treated as a motion artifact degrading the image quality. Conversely, respiratory motion in the thorax can be used to answer important clinical questions, that cannot be assessed adequately via conventional static thoracic CT, by utilizing four-dimensional (4D) CT. However, clinical experiences of 4D thoracic CT are quite limited. In order to use 4D thoracic CT properly, imagers should understand imaging techniques, radiation dose optimization methods, and normal as well as typical abnormal imaging appearances. In this article, the imaging techniques of pediatric thoracic 4D CT are reviewed with an emphasis on radiation dose. In addition, several clinical applications of pediatric 4D thoracic CT are addressed in various thoracic functional abnormalities, including upper airway obstruction, tracheobronchomalacia, pulmonary air trapping, abnormal diaphragmatic motion, and tumor invasion. One may further explore the clinical usefulness of 4D thoracic CT in free-breathing children, which can enrich one's clinical practice.

Semiautomatic Three-Dimensional Threshold-Based Cardiac Computed Tomography Ventricular Volumetry in Repaired Tetralogy of Fallot: Comparison with Cardiac Magnetic Resonance Imaging

OBJECTIVE: To assess the accuracy and potential bias of computed tomography (CT) ventricular volumetry using semiautomatic three-dimensional (3D) threshold-based segmentation in repaired tetralogy of Fallot, and to compare them to those of two-dimensional (2D) magnetic resonance imaging (MRI). MATERIALS AND METHODS: This retrospective study evaluated 32 patients with repaired tetralogy of Fallot who had undergone both cardiac CT and MRI within 3 years. For ventricular volumetry, semiautomatic 3D threshold-based segmentation was used in CT, while a manual simplified contouring 2D method was used in MRI. The indexed ventricular volumes were compared between CT and MRI. The indexed ventricular stroke volumes were compared with the indexed arterial stroke volumes measured using phase-contrast MRI. The mean differences and degrees of agreement in the indexed ventricular and stroke volumes were evaluated using Bland-Altman analysis. RESULTS: The indexed end-systolic (ES) volumes showed no significant difference between CT and MRI (p > 0.05), while the indexed end-diastolic (ED) volumes were significantly larger on CT than on MRI (93.6 ± 17.5 mL/m² vs. 87.3 ± 15.5 mL/m² for the left ventricle [p < 0.001] and 177.2 ± 39.5 mL/m² vs. 161.7 ± 33.1 mL/m² for the right ventricle [p < 0.001], respectively). The mean differences between CT and MRI were smaller for the indexed ES volumes (2.0–2.5 mL/m²) than for the indexed ED volumes (6.3–15.5 mL/m²). CT overestimated the stroke volumes by 14–16%. With phase-contrast MRI as a reference, CT (7.2–14.3 mL/m²) showed greater mean differences in the indexed stroke volumes than did MRI (0.8–3.3 mL/m²; p < 0.005). CONCLUSION: Compared to 2D MRI, CT ventricular volumetry using semiautomatic 3D threshold-based segmentation provides comparable ES volumes, but overestimates the ED and stroke volumes in patients with repaired tetralogy of Fallot.

Changes in Right Ventricular Volume, Volume Load, and Function Measured with Cardiac Computed Tomography over the Entire Time Course of Tetralogy of Fallot

OBJECTIVE: To characterize the changes in right ventricular (RV) volume, volume load, and function measured with cardiac computed tomography (CT) over the entire time course of tetralogy of Fallot (TOF). MATERIALS AND METHODS: In 374 patients with TOF, the ventricular volume, ventricular function, and RV volume load were measured with cardiac CT preoperatively (stage 1), after palliative operation (stage 2), after total surgical repair (stage 3), or after pulmonary valve replacement (PVR) (stage 4). The CT-measured variables were compared among the four stages. After total surgical repair, the postoperative duration (POD) and the CT-measured variables were correlated with each other. In addition, the demographic and CT-measured variables in the early postoperative groups were compared with those in the late postoperative and the preoperative group. RESULTS: Significantly different CT-based measures were found between stages 1 and 3 (indexed RV end-diastolic volume [EDV], 63.6 ± 15.2 mL/m2 vs. 147.0 ± 38.5 mL/m2 and indexed stroke volume (SV) difference, 7.7 ± 10.3 mL/m2 vs. 32.2 ± 16.4 mL/m2; p < 0.001), and between stages 2 and 3 (indexed RV EDV, 72.4 ± 19.7 mL/m2 vs. 147.0 ± 38.5 mL/m2 and indexed SV difference, 5.7 ± 13.1 mL/m2 vs. 32.2 ± 16.4 mL/m2; p < 0.001). After PVR, the effect of RV volume load (i.e., indexed SV difference) was reduced from 32.2 mL/m2 to 1.7 mL/m2. Positive (0.2 to 0.8) or negative (−0.2 to −0.4) correlations were found among the CT-based measures except between the RV ejection fraction (EF) and the RV volume load parameters. With increasing POD, an early rapid increase was followed by a slow increase and a plateau in the indexed ventricular volumes and the RV volume load parameters. Compared with the preoperative data, larger ventricular volumes and lower EFs were observed in the early postoperative period. CONCLUSION: Cardiac CT can be used to characterize RV volume, volume load, and function over the entire time course of TOF.

Computed Tomography Pulmonary Vascular Volume Ratio Can Be Used to Evaluate the Effectiveness of Pulmonary Angioplasty in Peripheral Pulmonary Artery Stenosis

OBJECTIVE: To explore whether computed tomography (CT) pulmonary vascular volume ratio can be used to evaluate the effectiveness of pulmonary artery angioplasty in patients with peripheral pulmonary artery stenosis. MATERIALS AND METHODS: Changes in CT pulmonary vascular volume ratio between serial cardiothoracic CT examinations were calculated in 38 patients. Fifteen patients underwent interim pulmonary artery angioplasty (group 1), while 23 did not (group 2). According to the effectiveness of pulmonary artery angioplasty, patients in group 1 were further divided into group 1A (improved or aggravated) and group 1B (ineffective). Changes in the pulmonary vascular volume percentages among the three groups (group 1A, group 1B, and group 2) on serial CT examinations were compared. RESULTS: Pulmonary artery angioplasty on serial CT examinations was successful in seven patients, ineffective in seven patients, and aggravated in one patient. As a result, eight patients were included in group 1A and seven were included in group 1B. Changes in the CT pulmonary vascular volume percentages in group 1A were statistically significantly greater than those in group 1B (11.6 ± 5.6% vs. 2.7 ± 1.6%, p 0.1). CONCLUSION: CT pulmonary vascular volume ratio can be used to evaluate the effectiveness of pulmonary artery angioplasty in patients with peripheral pulmonary artery stenosis.

User-Friendly Vendor-Specific Guideline for Pediatric Cardiothoracic Computed Tomography Provided by the Asian Society of Cardiovascular Imaging Congenital Heart Disease Study Group: Part 1. Imaging Techniques

Optimal performance of pediatric cardiothoracic computed tomography (CT) is technically challenging and may need different approaches for different types of CT scanners. To meet the technical demands and improve clinical standards, a practical, user-friendly, and vendor-specific guideline for pediatric cardiothoracic CT needs to be developed for children with congenital heart disease (CHD). In this article, we have attempted to describe such guideline based on the consensus of experts in the Asian Society of Cardiovascular Imaging CHD Study Group. This first part describes the imaging techniques of pediatric cardiothoracic CT, and it includes recommendations for patient preparation, scan techniques, radiation dose, intravenous injection protocol, post-processing, and vendor-specific protocols.

CT-Based Essential Cardiac Anatomy for Radiology Residents to Understand Congenital Heart Disease

Congenital heart disease is often associated with severe clinical presentations demanding prompt imaging diagnosis and appropriate treatment. Therefore, radiologists, particularly radiology residents, should be familiar with essential cardiac anatomy in order to diagnose congenital heart disease. In our clinical practice, cardiac anatomy is commonly depicted on CT images acquired with state-of-the art CT imaging techniques; however, the related imaging findings may be overlooked due to lack of attention, experience, or knowledge. It is partly due to the fact that CT-based illustration of cardiac anatomy to help understand congenital heart disease is currently scarce. In this article, cardiac imaging planes; crucial anatomical landmarks; morphological features of cardiac chambers, septa, and valves; and connections of cardiac segments are illustrated using cardiac CT images to facilitate understanding of congenital heart disease.